Gastrointestinal angiodysplasia in three Saudi children

Angiodysplasia is a term used to describe distinct gastrointestinal mucosal ectasias that are not associated with cutaneous lesions, systemic vascular disease or a familial syndrome. Seventy-seven percent of angiodysplasia are located in the cecum and/or ascending colon. Fifteen percent are located in the jejunum and/or ileum and the remainder are distributed throughout the alimentary tract. Most commonly, the angiodysplastic lesions are typically seen in elderly patients of both genders, although gastric and duodenal lesions have been reported occasionally in subjects within the third decade of life. However, data on infants and children are scarce. We describe three cases (ages 7 days, 2 years, and 5 years) who presented to our unit with gastrointestinal bleeding. One of these patients developed moderate-to-severe symptoms and was blood-transfusion dependent. She was misdiagnosed as having inflammatory bowel disease and underwent a total colectomy and ileoanal anastomosis. The other two patients were managed conservatively for up to 5 years with no further bleeding.

A ngiodysplasia is the term used to describe dist t tinct gastrointestinal mucosal vascular ectasias that are not associated with cutaneous lesions, systemic vascular disease or a familial syndrome. These lesions may be flat, red spots (2t5 mm) or slightly raised. The lesions have many similarities to those of the telangiectasias, but angiodysplasia is not a compot t nent of a systemic, metabolic or hereditary disease with other manifestations. 1 Seventytseven percent of angiot t dysplasias are located in the cecum and/or ascending colon. Fifteen percent are located in the jejunum and/or ileum and the remainder are distributed throughout the alimentary tract. 2 The exact cause of angiodysplasias is unknown, but the widely accepted theory is that they are related to degenerative changes of the small blood vessels associated with aging. Other theories include longtterm local hypotoxygenation of the microcircut t lation from cardiac, vascular or pulmonary disease. 1 Mucosal hypoperfusion from cardiac disease was post t tulated to be the underlying cause for development of angiodysplasia, but echocardiogram studies indicated that only a few patients with angiodysplastic lesions had significant valvular heart disease such as aortic stet t nosis. 2 In many instances angiodysplasia disappear folt t lowing aortic valve replacement. 3 Roskell et al demont t

Gastrointestinal angiodysplasia in three Saudi children
Angiodysplasia is a term used to describe distinct gastrointestinal mucosal ectasias that are not associ-ated with cutaneous lesions, systemic vascular disease or a familial syndrome. Seventy-seven percent of angiodysplasia are located in the cecum and/or ascending colon. Fifteen percent are located in the je-junum and/or ileum and the remainder are distributed throughout the alimentary tract. Most commonly, the angiodysplastic lesions are typically seen in elderly patients of both genders, although gastric and duodenal lesions have been reported occasionally in subjects within the third decade of life. However, data on infants and children are scarce. We describe three cases (ages 7 days, 2 years, and 5 years) who presented to our unit with gastrointestinal bleeding. One of these patients developed moderate-to-severe symptoms and was blood-transfusion dependent. She was misdiagnosed as having inflammatory bowel disease and underwent a total colectomy and ileoanal anastomosis. The other two patients were man-aged conservatively for up to 5 years with no further bleeding. strated a relative deficiency of collagen type IV in mut t cosal vessels in angiodysplasia compared with controls. 4 Angiodysplasia has been reported in adults, but data on infants and children are scarce. We describe three cases that presented to our pediatric gastroenterology unit.

CASE 1
The first case was a 5tyeartold Saudi female who had severe, frequent bloody diarrhea for two years associt t ated with intermittent abdominal pain, poor appetite and failure to gain weight. At age of 3 years, she was treated at her local hospital for amoebic dysentery with antibiotics (sulfamethoxazolettrimethoprim and mett t ronidazole) with no obvious improvement, followed by eliminating dietary milk products with some imt t provement, but she later had a recurrence of symptoms. Sigmoidoscopy in the local hospital showed a normal looking mucosa and biopsies showed chronic nont specific colitis; subsequently, the patient was started on prednisolone empirically with good response, but whenever the steroid dose was tapered, she started to bleed again from the rectum. She was referred to our center as having steroidtdependent colitis for further management. Examination revealed a pale, nontthrivt t ing child with both weight and height below the fifth percentile for age. The rest of the systemic examination was normal. Her hemoglobin was 5.3 mg/dL (normal, 11.0t14.0 mg/dL), mean corpuscular volume was 64.1 fL (normal, 80.0t94.0 fL), mean corpuscular hemot t globin was 19.9 pg/cell (normal, 27.0t32.0 pg/cell), the erythrocyte sedimentation rate was 31 mm/h, and stool for occult blood was positive, serum albumin was 38 g/L (normal, 32t46 g/L), and total protein was 69 g/L (normal 65t81 g/L). Colonoscopy revealed tiny ant t giodysplastic lesions in the rectum, sigmoid colon, and the ascending colon ( Figure 1). The transverse colon and cecum again showed small hemorrhagic angiodyst t plastic lesions with typical fold processes of dilated sut t perficial mucosal capillaries. No ulcers or erosions were seen ( Figure 2). The upper gastrointestinal endoscopy was normal. The steroid was tapered and stopped. She became blood transfusiontdependent, requiring one transfusion every 3 to 4 weeks. With involvement of the whole colon with angiodysplastic lesions, a normal upper gastrointestinal endoscopy and with moderatet totsevere symptoms requiring multiple blood transfut t sion, the patient underwent total colectomy and ileot t anal anastomosis with a smooth posttoperative course. Histological examination was consistent with angiot t dysplastic lesions in different parts of the colon ( Figure  3). Followtup in the clinic up to 3 years posttsurgery showed that the patient had no more rectal bleeding. Her growth improved and her hemoglobin stabilized at >10 mg/dL.

CASE 2
A 2tyeartold male was admitted at 5 months of age with fever and bronchopneumonia. He was treated with ant t tibiotics and improved. Two weeks posttdischarge, the infant developed hematemesis and melena. He was het t modynamically stable with a hemoglobin of 6.2 mg/dL (normal, 11.0t14.0 mg/dL) and needed blood transfut t sion. The liver and renal function tests were unremarkt t able. Upper gastrointestinal endoscopy in a local host t pital revealed angiodysplasia at the greater curvature of the stomach with some oozing points. There were no esophageal varices and no ulcers. He was referred to our center with recurrent hematemesis for further managet t ment. At the age of 1 year, upper GI endoscopy at our center revealed a normal esophagus with few angiodyst t plastic lesions over the body of the stomach while the duodenum was normal. Colonoscopy showed dilated blood vessels consistent with angiodysplastic lesions from the rectum to the midtascending colon. He was treated conservatively, without surgical intervention. He had no further bleeding and was placed on iron therapy. At the age of 6 years, he was seen in the clinic and had a weight and height above the tenth percentile. His hemoglobin was 13.3 mg/dL while maintained on an iron supplement.

CASE 3
A 7tdaytold male infant, a product of a fulltterm in vit t tro fertilization pregnancy was delivered by lower segt t ment cesarean delivery secondary to fetal distress by a mother with primary infertility for six years. The baby was dusky after birth and was resuscitated with oxygen using an Ambu bag, but did not need intubation to ret t solve transient tachypnea of the newborn. He was fed with artificial feeds (cow' s milk formula). On day 3, he had a bloody stool, with no abdominal distension and audible bowel sounds. He did not appear septic and vital signs were normal. He was placed on nil per OS nothing by mouth status and started on intravenous ampicillin and gentamicin after appropriate cultures were taken. An abdominal xtray was unremarkable. His complete blood count, including platelet count and coagulation profiles were normal. Stools on day 6, 7 and 8 were positive for occult blood. He underwent colonoscopy on day 8, which revealed angiodysplastic lesions oozing blood. The lesions increased in number as the scope advanced proximally to the sigmoid and descending colon and decreased toward the transverse and ascending colon. Bleeding appeared whenever air or water were applied directly over the lesions. The mother refused upper GI endoscopy for evaluation of the rest of the gut. The patient was treated with oral iron supplet t mentation and discharged home. On followtup, he had no more bleeding and received iron therapy as needed. At 3 years of followtup, the baby was growing well with no rectal bleeding and had a stable hemoglobin of 11 mg/dL.

DiSCuSSion
Angiodysplasia is an increasingly recognized disorder in adults and the prevalence appears higher than was originally suspected. 5 Among adults, angiodysplasia of the stomach or duodenum was found in 1% to 2% of 25 consecutive subjects undergoing upper gastrointestinal endoscopy for a variety of indications. 5 Angiodysplasia is found during colonoscopy at slightly higher rate of 3% to 6% of subjects undergoing the procedure for a variety of indications. 6,7 Angiodysplasia can be an incit t dental finding in nearly half of the cases in which it is detected. 6 Most commonly, the angiodysplastic lesions are typically seen in elderly patients of both genders, alt t though gastric and duodenal lesions occasionally have been reported in subjects during the third decade of life. 5,8 Data are scarce in infants and children, and to our knowledge, these are the first case reports among Saudi children. As with upper tract lesions, very young subjects with colonic angiodysplasia have also been det t scribed. 9 Angiodysplasia may present with maroontcolt t ored stool, melena, and hematochasia as in Case 1 and 3 or hematenesis as in Case 2. In 10% to 15% of patients, iron deficiency anemia is observed and stools are intert t mittently positive for occult blood (as in Case 2 and 3). 6 Fifteen percent of patients present with massive hemort t rhage as in Case 1, and bleeding stops spontaneously in more than 90% of cases (as in Case 2 and 3). 7 While there are many modalities for diagnosis of angiodysplat t sia such as selective mesenteric angiography, 99m Tct labeled red blood cells, 99m Tctlabeled sulfur colloid, CT angiography, air contrast enema and wireless capt t sule, colonoscopy is the most common method of diagt t nosis as well as therapy. The sensitivity of colonoscopy exceeds 80% when the lesions are located in the area examined by colonoscopy. 7 In Case 1, a limited sigmoidoscopy was performed and she was misdiagnosed as having inflammatory bowel disease. Endoscopic forceps biopsy has revealed characteristic histopathological features of angiodysplat t sia in only 31% to 60% of specimens. 10 Angiodysplasia is infrequently detected by visual inspection of the serosal side of the bowel wall. 11 In 1 of 39 adult cases (2.6%), the diagnosis was made during surgical exploration, and 14 (35.8%) of these individuals underwent 21 nontdit t agnostic surgeries prior to their evaluation. A consert t vative approach to hemodynamically stable patients is